HELLP Syndrome: A Rare Cause of Abdominal Pain in Pregnancy
Pregnancy is one of the most delicate times in a woman’s life. It is also a critical phase since not only is the mother’s health is at risk but also that of the unborn fetus. Concurrently, there are many changes in the body that may seem abnormal, especially for first-time mothers.
One of the common problems that cause major stress is abdominal pain. There are several disease entities that may cause distress in mothers with abdominal pain in pregnancy; herewith is a case of a very dangerous and rare condition that may prove fatal if not properly diagnosed early on.
As an introduction to this disease, we have presented a case of a twenty-eight-year-old woman who came in due to abdominal pain. The patient was in distress at the time of consult. She noted that this was her first pregnancy and also the first time to go to a hospital for prenatal checkup. There were no prior records of the patient nor the fetus. Upon examination, she was pale, in distress with elevated heart rate, respiratory rate, and slightly decreased blood pressure. Given the situation, what disease entities may have caused her condition?
HELLP syndrome occurs in 0.1%-0.6% of all pregnancies and in 4%-12% of patients with preeclampsia. HELLP syndrome typically occurs between week 27 of gestation and delivery, or immediately postpartum in 15%-30% of cases.— American College of Obstetricians and Gynecologists
HELLP is a life-threatening obstetric complication and an abbreviation for its three main features:
- Elevated Liver Enzymes
- Low Platelet Count
It occurs in about 0.2 to 0.8% of all pregnancies (Abildgaard & Heimdal, 2013), in 10% of preeclampsia cases and in 50% of eclampsia cases (Stella, et. al, 2007). Pre-eclampsia and eclampsia are commonly known in laymen as hypertension during pregnancy. This may or may not present in patients who are already having hypertension. A number of references claim that HELLP is a variant, a complication or a severe form of preeclampsia implying its presence as a diagnostic requirement. Some authors say, however, that HELLP is a separate syndrome only with overlapping features with preeclampsia, in fact, as many as 15 to 20% of HELLP patients do not have antecedent hypertension or proteinuria (Satpathy, et. al, 2009). A brief discussion of the pathogenesis of HELLP as well as its parallelism to the case assigned follows.
In order to diagnose this condition, there are several criteria medical practitioners use. Some of which are introduced in the succeeding statements. According to the review published by Abildgaard & Heimdal (2013), there are two main diagnostic definitions of the HELLP syndrome: The more widely used Tennessee classification and the Mississippi Triple-class classification. The Tennessee classification requires presence of:
- Laboratory findings consistent with microangiopathic hemolytic anemia, abnormal peripheral smear, elevated serum indirect bilirubin, low serum haptoglobin and elevated LDH levels.
- Elevated liver enzymes: aspartate aminotransferase (ASAT) levels above 70 IU/L,lactate dehydrogenase (LD) above 600 IU/L and abnormal AST and ALT levels above 17 to 72 IU/L (although in some references the clear-cut levels aren’t defined).
- Platelet count below 100 x 109/L.
The Mississippi Triple classification further classifies the disorder according to the levels of the platelet count.
What causes this condition and what mechanisms lead to the signs and symptoms presented by patients?
Although most information about the pathogenesis leading to the causation and clinical manifestations of the HELLP syndrome remains largely unknown, this syndrome generally develops suddenly between 28-36 weeks of gestation and is generally accepted to be placenta-instigated (or at least a major causative factor) and involves an aberrant immunological process with an angiopathy having a central role (Abildgaard & Heimdal, 2013). Like in preeclampsia, it results from abnormal development and function of the placenta (including uterine placental spiral arterioles) leading to its ischemia and necrosis thereby releasing inflammatory factors such as reactive oxygen species and lipid peroxides that negatively influence intrauterine fetal growth and development. Inflammatory factors from the placenta also contribute to the injury of the endothelium and an activation of the coagulation cascade. Arteriolar occlusion then results from platelet and fibrin microthrombi leading to a thrombotic microangiopathy, characterized by destruction of red blood cells as if they were being forced through a strainer. The same fibrin deposits occurring in hepatic sinusoids causes obstruction of hepatic outflow eventually leading to the elevation of liver enzymes and periportal necrosis. The decrease in platelet count may be attributed to increased consumption and destruction of platelets as in disseminated intravascular coagulation (compensated in most cases) that is promoted by the abovementioned thrombotic microangiopathy (caused by inflammatory factors) (Satpathy, et. al, 2009).
How severe is this disease? Can pregnant women actually lose children or even die with this condition?
HELLP-syndrome associated severe and potentially fatal complications include disseminated intravascular coagulation, abruptio placentae, acute renal failure, pulmonary edema, and hepatic subcapsular hematoma. Hepatic hemorrhages/rupture are the most rare (0.9-2% of cases) but also most severe of all the complications, with a mortality rate of 18-86% (Kapana, et al., 2010). Progressive bleeding leads to accumulation of blood under the Glisson’s capsule, which under severe cases, leads to hepatic rupture causing hemoperitoneum leading to hypovolemic shock. Patients affected are usually multiparous like the patient in this case and most frequently happens just prior to delivery or postpartum (Matheï, 2007). Clinical, laboratory and imaging studies (serial ultrasound) help support this diagnosis. Signs of hepatic enlargement, epigastric or RUQ pain are classically present. The laboratory findings are characteristic of HELLP syndrome or DIC as seen in the patient. Rupture typically produces a triad of preeclampsia or eclampsia, abdominal pain, and hypotension, with pathological examination revealing sinusoidal fibrin deposition and extensive periportal, hemorrhage (Cappell and Friedel, 2003).
Clinical Case Explained
Laboratory findings and some clinical findings in the case assigned, albeit incomplete to make a definitive diagnosis (based on the criteria discussed above), support at least the consideration of HELLP.
Following the gestational age confirmed by ultrasound (32 6/7 weeks), the onset of maternal symptoms are concurrent with the expected onset of HELLP (28 to 36 weeks of gestation). The presence of an abnormal low lying placenta early in the pregnancy as seen in the patient is also observed, in some cases, as an antecedent to the diagnosis of HELLP. The intrauterine fetal growth retardation and eventual intrauterine fetal death could be attributed to the abnormalities in the placenta causing ischemia. Five hours post-partum, the patient complained of epigastric pain which is seen in 65% of HELLP cases (Satpathy, et. al, 2009). On physical examination following complaint of epigastric pain, the patient was found to be hypotensive, tachycardic and tachypneic with pale palpebral conjunctiva, all attributable to a massive blood loss which can be attributed to a hepatic hemorrhage leading possibly leading to capsular tear and hemoperitoneum. This means that during the time of consult, the patient was already possibly bleeding that’s why there was abdominal pain. Also, the vital signs were already deranged and the patient was pale leading to a stipulation that the patient was already having severe bleeding.
On CBC following these sign and symptoms, platelet count was found to be very low, fulfilling the third criterion of the Tennessee classification. Subsequent blood chemistry examination revealed extremely elevated levels of AST and ALT, complying with the second criterion. Although, a laboratory test that could confirm the presence of microangiopathic hemolytic anemia (first criterion) are not available, a high suspicion for HELLP is still justifiable due to the similarities in the case and the common presenting features of the disease.
The progression of the pathogenesis of hepatic hemorrhage leading to hepatic subcapsular hematoma associated with HELLP syndrome is not yet fully known. Current thought stipulates this is secondary to fibrin thrombus formation, with a predilection for the liver arterioles and sinusoid capillaries. This process eventually leads to periportal necrosis, intrahepatic hemorrhage, subcapsular liver hematoma, or liver rupture, with most cases occurring in the right hepatic lobe (Cappell and Friedel, 2003).
Management of this complication includes conservative management with steroids and transfusion in hemodynamically stable patients. For patients presenting with shock, the emergency surgical procedure including the evacuation of the hematoma with packing and drainage, angiographic embolization, hepatic artery ligation, or hepatic resection (Cappell and Friedel, 2003).
- Abildgaard, U., Heimdal, K. (2013). Pathogenesis of the syndrome of hemolysis, elevated liver enzymes, and low platelet count (HELLP): a review. European Journal of Obstetrics & Gynecology and Reproductive Biology. 166; 117-123
- Cappell MS1, Friedel D. (2003). Abdominal pain in pregnancy. Gastroenterol Clin North Am. 32(1):1-58.
- Chen, H., Yuan, L., Tan, J., Liu, Y., & Zhang, J. (2008). Severe liver disease in pregnancy. International Journal of Gynecology & Obstetrics, 101(3), 277–280. doi:10.1016/j.ijgo.2007.12.011
- Satpathy, H.K., Satpathy, C., Frey, D. (2009). Review Article: HELLP Syndrome. Journal of Obstetric and Gynecology of India. 59 (1); 30-40
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© 2016 LM Gutierrez